Background/aim: Childhood interstitial lung diseases (chILD) constitute a rare and clinically complex group of disorders. This study aimed to characterize the clinical, radiological, and genetic features, as well as the outcomes, of chILD in a Turkish cohort classified according to the chILD-EU framework. Materials and methods: We retrospectively reviewed the medical records of 84 pediatric patients diagnosed with chILD between 2017 and 2024 at a tertiary referral center in Türkiye. Patients were categorized according to the chILD-EU classification. Clinical variables, imaging findings, genetic analyses, pulmonary function test results, and Fan severity scores were systematically assessed. Logistic regression analysis was performed to identify independent predictors of clinical instability. Results: The median age at diagnosis was 6.0 years (IQR: 1.1–12.9). Surfactant dysfunction disorders (A4) and immune- or environmental-related diseases (B2) were the most frequently identified subtypes. Hypoxia was observed in 36 of 84 patients (42.8%) and emerged as the strongest independent predictor of clinical instability (OR: 8.5; 95% CI: 2.2–33.0; p = 0.002). Pathogenic or likely pathogenic variants were identified in 18 of 84 patients (21.4%); among variant-positive cases, ABCA3 was the most frequently affected gene (3 of 18; 16.7%). Chest computed tomography was available in 82 of 84 patients, with ground-glass opacities being the most common finding, observed in 51 of 82 patients (62.2%). A decrease of at least one point in the Fan severity score was observed in 42 of 84 patients (p < 0.001). Mortality was 12 of 84 patients (14.3%) after a median follow-up of 3.2 years (range: 1.2–4). Conclusion: This study presents one of the largest single-center pediatric chILD cohorts reported from Türkiye. It highlights the prognostic relevance of baseline hypoxia and underscores the importance of comprehensive radiological and genetic assessment in the management of chILD.